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Profile

Jaime Vengoechea Barrios

  • Department of Human Genetics
    Associate Professor
  • jvengoe@emory.edu
  • 1365 Clifton Road, NE
    Building A, Suite 1500
    Atlanta, GA 30322
Head shot of Jaime Vengoechea Barrios

Overview

I am a Clinical Geneticist with the Division of Medical Genetics. I specialize in clinical genetics for adults, including inherited cancer syndromes, cardiovascular disorders, and hereditary neurologic disorders.

Academic Appointment

  • Associate Professor, Emory University

Education

Degrees

  • Residency, Combined Internal Medicine and Medical Genetics Programs from Case Western Reserve University
  • MD from Pontificia Universidad Javeriana

Research

Publications

  • Switching treatment to cipaglucosidase alfa plus miglustat positively affects patient-reported outcome measures in patients with late-onset Pompe disease.
    J Patient Rep Outcomes Volume: 8 Page(s): 132
    11/13/2024 Authors: Kishnani PS; Byrne BJ; Claeys KG; Daz-Manera J; Dimachkie MM; Kushlaf H; Mozaffar T; Roberts M; Schoser B; Hummel N
  • PSMD11 loss-of-function variants correlate with a neurobehavioral phenotype, obesity, and increased interferon response.
    Am J Hum Genet Volume: 111 Page(s): 1352 - 1369
    07/11/2024 Authors: Deb W; Rosenfelt C; Vignard V; Papendorf JJ; Mller S; Wendlandt M; Studencka-Turski M; Cogn B; Besnard T; Ruffier L
  • Clinical and biological landscape of constitutional mismatch-repair deficiency syndrome: an International Replication Repair Deficiency Consortium cohort study.
    Lancet Oncol Volume: 25 Page(s): 668 - 682
    05/01/2024 Authors: Ercan AB; Aronson M; Fernandez NR; Chang Y; Levine A; Liu ZA; Negm L; Edwards M; Bianchi V; Stengs L
  • Isolated cardiomyopathy in a pathogenic X-linked in frame hemizygous DMD exon 49 deletion: A rare presentation with normal creatine kinase levels and absence of musculoskeletal symptoms.
    Am J Med Genet A Volume: 194 Page(s): e63475
    03/01/2024 Authors: Aljaberi R; Vengoechea J
  • Biallelic variants in GTF3C5, a regulator of RNA polymerase III-mediated transcription, cause a multisystem developmental disorder.
    Hum Genet Volume: 143 Page(s): 437 - 453
    03/01/2024 Authors: Iwata-Otsubo A; Skraban CM; Yoshimura A; Sakata T; Alves CAP; Fiordaliso SK; Kuroda Y; Vengoechea J; Grochowsky A; Ernste P
  • Autosomal Recessive Adolescent Syndromic Nephronophthisis Caused by a Novel Compound Heterozygous Pathogenic Variant.
    Am J Case Rep Volume: 24 Page(s): e941413
    11/22/2023 Authors: Ajiboye O; Vengoechea JE; Gupta R; Lomashvili K
  • De novo missense variants in the E3 ubiquitin ligase adaptor KLHL20 cause a developmental disorder with intellectual disability, epilepsy, and autism spectrum disorder.
    Genet Med Volume: 24 Page(s): 2464 - 2474
    12/01/2022 Authors: Sleyp Y; Valenzuela I; Accogli A; Ballon K; Ben-Zeev B; Berkovic SF; Broly M; Callaerts P; Caylor RC; Charles P
  • Variant-specific changes in RAC3 function disrupt corticogenesis in neurodevelopmental phenotypes.
    Brain Volume: 145 Page(s): 3308 - 3327
    09/14/2022 Authors: Scala M; Nishikawa M; Ito H; Tabata H; Khan T; Accogli A; Davids L; Ruiz A; Chiurazzi P; Cericola G
  • THINKING OUTSIDE THE MUSCLE: NON-NEUROLOGICAL CLUES IN INHERITED MYOPATHIES, TWO CASE REPORTS
    Volume: 66 Page(s): S32 - S32
    09/01/2022 Authors: De Leon A; Dhir A; Vengoechea J; Santibanez RG
  • Safety and efficacy of cipaglucosidase alfa plus miglustat versus alglucosidase alfa plus placebo in late-onset Pompe disease (PROPEL): an international, randomised, double-blind, parallel-group, phase 3 trial.
    Lancet Neurol Volume: 20 Page(s): 1027 - 1037
    12/01/2021 Authors: Schoser B; Roberts M; Byrne BJ; Sitaraman S; Jiang H; Lafort P; Toscano A; Castelli J; Daz-Manera J; Goldman M
  • Identical twins with progressive kyphoscoliosis and ophthalmoplegia: Expert commentary.
    Parkinsonism Relat Disord Volume: 92 Page(s): 123 - 124
    11/01/2021 Authors: Vengoechea J; Li H; Jinnah HA
  • Two females with distinct de novo missense pathogenic variants in MED12 and vastly differing phenotypes.
    Am J Med Genet A Volume: 185 Page(s): 2582 - 2585
    08/01/2021 Authors: Gonzalez A; Kapur S; Walsh M; Vengoechea J
  • Recurrent Coronary Artery Fistulae and a Novel TGBR3 Mutation: A Case Report
    Volume: 203
    05/01/2021 Authors: Abdelhadi NA; Alraies M; Sattar Y; Vengoechea J; Jokhadar M
  • ClinGen's actionability working groups: development and early implementation of an assertion rubric for clinical actionability
    Volume: 132 Page(s): S125 - S126
    04/01/2021 Authors: Pak C; Hunter JE; Gilmore M; Lee K; Mittendorf K; Wallace K; Bulkley J; Jenkins C; Dagan-Rosenfeld O; Foreman AK
  • Absent B cells, agammaglobulinemia, and hypertrophic cardiomyopathy in folliculin-interacting protein 1 deficiency.
    Blood Volume: 137 Page(s): 493 - 499
    01/28/2021 Authors: Saettini F; Poli C; Vengoechea J; Bonanomi S; Orellana JC; Fazio G; Rodriguez FH; Noguera LP; Booth C; Jarur-Chamy V
  • Association of metabolic syndrome with glioblastoma: a retrospective cohort study and review.
    Neurooncol Pract Volume: 7 Page(s): 541 - 548
    10/01/2020 Authors: Rogers LR; Ostrom QT; Schroer J; Vengoechea J; Li L; Gerson S; Nock CJ; Machtay M; Selman W; Lo S
  • A case of Singleton-Merten syndrome without cardiac involvement harboring a novel IFIH1 variant.
    Am J Med Genet A Volume: 182 Page(s): 1535 - 1536
    06/01/2020 Authors: Vengoechea J; DiMonda J
  • Combined occurrence of deleterious TOR1A and ANO3 variants in isolated generalized dystonia.
    Parkinsonism Relat Disord Volume: 73 Page(s): 55 - 56
    04/01/2020 Authors: Miocinovic S; Vengoechea J; LeDoux MS; Isbaine F; Jinnah HA
  • Segregation of two variants suggests the presence of autosomal dominant and recessive forms of WFS1-related disease within the same family: expanding the phenotypic spectrum of Wolfram Syndrome.
    J Med Genet Volume: 57 Page(s): 121 - 123
    02/01/2020 Authors: Lusk L; Black E; Vengoechea J
  • A rare mutation in hypophosphatasia: a case report of adult form and review of the literature.
    Arch Endocrinol Metab Volume: 63 Page(s): 89 - 93
    02/01/2019 Authors: Galeano-Valle F; Vengoechea J; Galindo RJ
  • In reply to "Mast Cell Disorders in Ehlers-Danlos Syndrome".
    Am J Med Genet A Volume: 176 Page(s): 250
    01/01/2018 Authors: Vengoechea J
  • A germline deletion of 9p21.3 presenting as familial melanoma, astrocytoma and breast cancer: clinical and genetic counselling challenges.
    J Med Genet Volume: 54 Page(s): 682 - 684
    10/01/2017 Authors: Vengoechea J; Tallo C
  • A splice site mutation in ATP6AP2 causes X-linked intellectual disability, epilepsy, and parkinsonism.
    Parkinsonism Relat Disord Volume: 21 Page(s): 1473 - 1475
    12/01/2015 Authors: Gupta HV; Vengoechea J; Sahaya K; Virmani T
  • Personalized Medicine: Monogenic Diabetes.
    J Ark Med Soc Volume: 112 Page(s): 58 - 59
    09/01/2015 Authors: Goulden PA; Vengoechea J; McKelvey K
  • Gnathodiaphyseal dysplasia presenting as polyostotic fibrous dysplasia.
    Am J Med Genet A Volume: 167 Page(s): 1421 - 1422
    06/01/2015 Authors: Vengoechea J; Carpenter L
  • Cholesterol and family history: when genetics matters.
    J Ark Med Soc Volume: 111 Page(s): 184 - 186
    02/01/2015 Authors: Vengoechea J; McKelvey KD
  • Papillary thyroid cancer in a patient with interstitial 6q25 deletion including ARID1B.
    Am J Med Genet A Volume: 164A Page(s): 1857 - 1859
    07/01/2014 Authors: Vengoechea J; Carpenter L; Zrate YA
  • Molecular subtypes of glioblastoma are relevant to lower grade glioma.
    PLoS One Volume: 9 Page(s): e91216
    01/01/2014 Authors: Guan X; Vengoechea J; Zheng S; Sloan AE; Chen Y; Brat DJ; O'Neill BP; de Groot J; Yust-Katz S; Yung W-KA
  • Description of the first case of adenomyomatosis of the gallbladder in an infant.
    Case Rep Pediatr Volume: 2014 Page(s): 248369
    01/01/2014 Authors: Zarate YA; Bosanko KA; Jarasvaraparn C; Vengoechea J; McDonough EM
  • Duplication of the ALDH1A2 gene in association with pentalogy of Cantrell: a case report.
    J Med Case Rep Volume: 7 Page(s): 287
    12/30/2013 Authors: Steiner MB; Vengoechea J; Collins RT
  • Clinical variability and female penetrance in X-linked familial FTD/ALS caused by a P506S mutation in UBQLN2.
    Amyotroph Lateral Scler Frontotemporal Degener Volume: 14 Page(s): 615 - 619
    12/01/2013 Authors: Vengoechea J; David MP; Yaghi SR; Carpenter L; Rudnicki SA
  • Methylation markers of malignant potential in meningiomas.
    J Neurosurg Volume: 119 Page(s): 899 - 906
    10/01/2013 Authors: Vengoechea J; Sloan AE; Chen Y; Guan X; Ostrom QT; Kerstetter A; Capella D; Cohen ML; Wolinsky Y; Devine K
  • Genetics and cardiovascular disease: the impact of molecular diagnosis.
    J Ark Med Soc Volume: 109 Page(s): 230 - 232
    04/01/2013 Authors: Vengoechea J; McKelvey KD
  • A variable age of onset segregation model for linkage analysis, with correction for ascertainment, applied to glioma.
    Cancer Epidemiol Biomarkers Prev Volume: 21 Page(s): 2242 - 2251
    12/01/2012 Authors: Sun X; Vengoechea J; Elston R; Chen Y; Amos CI; Armstrong G; Bernstein JL; Claus E; Davis F; Houlston RS
  • De novo microduplication of the FMR1 gene in a patient with developmental delay, epilepsy and hyperactivity.
    Eur J Hum Genet Volume: 20 Page(s): 1197 - 1200
    11/01/2012 Authors: Vengoechea J; Parikh AS; Zhang S; Tassone F
  • ASSOCIATION BETWEEN GLIOBLASTOMA MOLECULAR SUBTYPES AND LOWER-GRADE GLIOMAS
    Volume: 14 Page(s): 92 - 92
    10/01/2012 Authors: Guan X; Vengoechea J; Zheng S; Sloan A; Chen Y; Brat D; O'Neill BP; Cohen M; Aldape K; Rosenfeld S
  • ASSOCIATION BETWEEN HISTORY OF CHICKENPOX AND/OR SHINGLES AND SURVIVAL IN GLIOBLASTOMA
    Volume: 14 Page(s): 23 - 23
    10/01/2012 Authors: Rotman LE; Ostrom Q; Vengoechea J; Kuhns B; Rogers L; Sloan A; Barnholtz-Sloan J
  • FREQUENCY OF THE METABOLIC SYNDROME AND ASSOCIATION WITH SURVIVAL IN NEWLY DIAGNOSED GBM PATIENTS: A RETROSPECTIVE REVIEW
    Volume: 14 Page(s): 21 - 21
    10/01/2012 Authors: Rogers LR; Ostrom Q; Vengoechea J; Chen Y; Davitkov P; Strodtbeck K; Selman WR; Gerson S; Nock C; Machtay M
  • A nomogram for individualized estimation of survival among patients with brain metastasis.
    Neuro Oncol Volume: 14 Page(s): 910 - 918
    07/01/2012 Authors: Barnholtz-Sloan JS; Yu C; Sloan AE; Vengoechea J; Wang M; Dignam JJ; Vogelbaum MA; Sperduto PW; Mehta MP; Machtay M
  • Germline mosaicism in Cornelia de Lange syndrome.
    Am J Med Genet A Volume: 158A Page(s): 1481 - 1485
    06/01/2012 Authors: Slavin TP; Lazebnik N; Clark DM; Vengoechea J; Cohen L; Kaur M; Konczal L; Crowe CA; Corteville JE; Nowaczyk MJ
  • Proteomics signature of long-term versus short-term survival in glioblastoma
    CANCER RESEARCH Volume: 72
    04/15/2012 Authors: Barnholtz-Sloan J; Gokulrangan G; Chen Y; Yohannes E; Vengoechea J; Sloan AE; Chance M
  • Frequency of the Metabolic Syndrome and Association with Survival in Newly Diagnosed GBM Patients: A Retrospective Review
    Volume: 78
    04/01/2012 Authors: Rogers L; Vengoechea J; Ostrom Q; Chen Y; Davitkov P; Strodtbeck K; Selman W; Gerson S; Nock C; Machtay M
  • Variation in age at cancer diagnosis in familial versus nonfamilial Barrett's esophagus.
    Cancer Epidemiol Biomarkers Prev Volume: 21 Page(s): 376 - 383
    02/01/2012 Authors: Chak A; Chen Y; Vengoechea J; Canto MI; Elston R; Falk GW; Grady WM; Guda K; Kinnard M; Markowitz S
  • Family history of cancer in benign brain tumor subtypes versus gliomas.
    Front Oncol Volume: 2 Page(s): 19
    01/01/2012 Authors: Ostrom QT; McCulloh C; Chen Y; Devine K; Wolinsky Y; Davitkov P; Robbins S; Cherukuri R; Patel A; Gupta R
  • Misconduct in medical students.
    Dev World Bioeth Volume: 8 Page(s): 219 - 225
    12/01/2008 Authors: Vengoechea J; Moreno S; Ruiz A
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